Welcome To My Blog...

This blog follows my journey of 2 different cochlear implants and my condition: Multiple mitochondrial DNA deletions I have started this blog 15 yrs too late but ill try my best to fit it all in! I have packed a lot of medical jargon into my life since I was 8.

There has been happiness and tears but I've come through it all with my family and my friends.I'm profoundly deaf as a result of a condition called Multiple mitochondrial DNA deletions or mitochondrial disease RRM2B as my professor Sir Dough Turnbull calls it! I have had since birth but I didn't find out this til I was 19. I have had 2 cochlear implants (at the age of 8 and then i lost the 1st cochlear implant in my right ear after 7 years due to a bad, accruing ear infection (which I couldnt fight off because of my mitochondrial condition) at the age of 15 and had a 2nd one implanted in my left ear that same year which I have now.

My Story

31 July 2008

OCD (obsessive compulsive disorder)

A few months after my intensive care stint in 2003, I found myself washing my hands a lot more. I had developed obsessive compulsive disorder I found it very hard to accept that I might have had Myasthenia gravis . until I later found out the fact I had Multiple mitochondrial DNA deletions Something clicked in my head and put me into a robot mode and I felt I HAD to wash my hands from time to time.

I blamed the cleanliness of intensive care unit because I felt if my hands were clean I wouldn’t get ill again or catch another ear infection and lose my cochlear implant. I was just freaked at the fact that I might lose my other cochlear implant and be in a silent world for the rest of my life. Although I knew I’m already profoundly deaf, I hated the fact I might lose my cochlear implant and never hear my family and friends voices or music etc again. I began being very protective about my ears and wouldn’t let anyone else touch them apart from my mum and my doctor. I began being more looser on the protective mode on the left ear because of my cochlear implant but I never let anyone touch my right ear because I felt I still had my ear infection and if anyone or anything touched my infected and touched my other ear, I would go into question mode - "is my ear ok?" "Is that ok?" In my case I keep saying "you promise?" Even now (2008) when I know my ear infection has healed, I still wont let anyone touch it.

I knew it was not normal of me to do so but I couldn’t help myself and felt I had to do it. I still have OCD and get very frustrated and wish I didn’t have it because it really gets on my nerves and can be time consuming. I get very angry at times and lash out because I know I shouldn’t be doing it and wish I was better. I began to look for help. My G.P put me in touch with a doctor about it. He recommended I try behavioural therapy. It helped for a bit but I found it hard because the lady I was working with was very judgemental towards me and told me I was being lazy! I felt so angry about that and I stopped it there and then!

I went back to the OCD doctor with my parents and the OCD doctor suggested trying
Prozac, he told me it would be 4 weeks before it started working. I began taking Prozac and after a month on them, one evening I felt my lip getting big and puffy. It wasn't till my parents came home from a night out that I noticed something was wrong because my mum was shocked when she saw me. She rang the Medical helpline on the phone for advice. The advice was to keep an eye on me and if it was no better in the morning see a doctor but as it was a Saturday night we would have to go to casualty. Overnight, it had got worse and my face had become very puffy and I had a rash all over my body. My eyes were closing up that I could hardly see.

My parents took me to casualty the next morning, I went there in my PJs, I was very scared and I was holding on to my mum because she was guiding me where to go. I saw a doctor after half an hour and he checked me out. We told him about the medication I was on but he told me I should keep taking it as it’s an anti depressant. He gave me some steroids to take home.

I took the steroids (they were pink dissolvable ones and yes they tasted horrible!!) and as soon as I got home and it seemed to get better but then that evening I took my Prozac as the doctor had told me too, it flared up again so we saw my GP the next day. She told me that it was obvious that the Prozac was the cause of the reaction and that casualty should have never told me to keep taking them! I stopped taking them and I began to get better. It turns out I was allergic to the SSRIs in the Prozac. We made a complaint about this.

When I went back to my OCD doctor we told what had happened and he gave me
Clomipramine instead. I wasn’t happy about being on medication as it’s what I wanted to avoid.

After a year on Clomipramine, I decided to stop it because I felt it wasn't helping my OCD. It was still the same as it ever was and there was no point of being on it. I began to get better for a bit. I have my good days and my bad days although now in 2008 it’s getting a bit worse so I have decided to try behavioural therapy again. I want to beat the OCD because I hate having it and it can be very frustrating at times, I get very angry about it. Hopefully one day will be free of it.

Multiple mitochondrial DNA deletions

In Summer 2004, I went to my appointment with my parents at Bradford and Mr Busby told us that I had "Multiple mitochondrial DNA deletions" a condition that affects my muscles and my energy genes.

He explained that it was the cause of my profound deafness, my fussiness with food was down to the fact I chose easier foods to eat because of my swallowing. It also affects my muscles and energy levels leaving me tired at times.
The ear infection I had in my right ear was down to the fact I wasn’t getting the right nutrients from the foods I was eating and my droopy eyelids were also a factor of the condition.
The main factor was my weight and how I could never put any on and after the month I had in hospital at Christmas, my weight was 6st and I was left very skinny for my age of 18.
I worried if I couldn’t put the weight back on and look a bit healthier.
The sample of my muscle biopsy Mr Busby had sent to Newcastle told us I may have had it since birth. Mr Busby thought it would be worth going to see Professor Turnbull, the top doc of my condition at Royal Victoria Infirmary at Newcastle upon Tyne and made an appointment for me to go see him
I had never been to Newcastle before and it was 3 hours from where we live! Quite a way to go to see a doctor for an appointment but Mr Busby assured us we would still be seeing himself between appointments at Newcastle for checkups.
So in May 2004 went up to Newcastle Victoria Infirmaty. We went to Metro Centre for a break and refreshments. Having got lost on the way to the hospital, we were slightly late for our appointment! We had never been in to the city centre of Newcastle before but my dad who is a wagon driver was very capable of finding it! We went in and met Mr Tunrbull, who was very nice and friendly. Mr Turnbull asked us a lot of questions regarding our family history like the fact my brother didn't have the condition, he also told us more about the condition I had. He sent me for a blood test which was right accross the other side of the hospital and came back to Mr Turnbull. He told us that it was very rare that someone of my age (20) was showing the signs of Multiple mitochondrial DNA deletions as this doesnt happen til you are in your 40s, It was all very intresting to know.

30 July 2008

The Muscle Biopsy

In January 2004, when I had an hospital appointment Mr Busby thought it was worth having a muscle biopsy to determine whether I had Myasthenia gravis because of the fact I was unable to put weight on, my deafness and droopy eyelids. I got quite worried about this because it sounded scary! I knew it had to be done though to find out what was wrong with me.
I went in for the usual routine pre- op things to check and meet the surgeon that would be doing my muscle biopsy. I wasn’t sure what to expect from a muscle biopsy but the surgeon told me all about it.

At the end of January, I was admitted to hospital for the muscle biopsy. My mum came with me for support. I wasn't looking forward to it and once I got into my hospital gown, I start to worry a lot. The surgeon came round to ask me which leg I would like the muscle biopsy on. I chose my left leg. He drew an arrow with purple pen on my leg to show where it would be. It looked funny having an arrow on my thigh!

When the bed came to take me, I burst into tears because it was so scary for me. My mum tried to calm me down and the surgeon told me if I didn’t calm down he would not be able to perform the muscle biopsy. I took a big breath and forced myself to calm down and get on the bed.
I got to theatre and they needed to give me a local anaesthetic in my thigh. Once the first one went in I screamed the hospital down because it was stinging so bad! I was wriggling about because of the pain so the surgeon took his chance and put a few locals in my leg and I nearly broke my mum's hand!! The combination of being upset and having the local was very traumatic! Once the local had begun to work and part of my thigh became numb, I settled down.
I felt cold because I was in a thin hospital gown and I began to shiver as they wheeled me in the theatre. The surgeon put a cover between my head and the rest of my body. A nurse began talking to me asking me questions about my life to distract me from what was happening.
I could feel tightness and some pulling but no pain which I was grateful for! It was all over in 20 minutes

I came back with a neat tight bandage around my thigh and I had no pain YET! I was told I could go home or stay the night at hospital. I opted to go home! The nurse told me if it got painful, I should take a painkiller or some Calpol. As I can't swallow tablets, I went for the Calpol! My dad came to pick me up from the hospital in the evening when he had finished work. My mum helped me get dressed and ready to go home. My dad stopped at the supermarket on the way home and mum bought me some Calpol.

I was relieved to get home and rest my leg on the sofa! It was painful going up the stairs to get to bed. My brother yet had to give up his bed for me and I was out like a light!
After 5 days I went back to the hospital to have the bandage taken off, when they took it off I was shocked to see 9 staples in my thigh. I was very relieved to get the bandage off as it was tight. The nurse gave it a clean and put a new dressing on it. I would be getting my staples out my local GP's surgery in a few days time!
I had the 9 staples taken out by a nifty gadget and it was painless! The nurse put another dressing on to let it heal. I now have a small scar on my left thigh! It’s quite cool and reminds me of what I went through and how brave I was!
The biopsy was send to Bradford and Newcastle hospital for testing.

Plasma exchange

Me in 2005, when i was 6st

When I got to the intensive care unit, the nurses were very nice and told me not to worry. As they wheeled me in, I saw lots of machines and people with tubes in them but I was too weak to be bothered by it. I just wanted to be better again.

Mr Busby decided to try a
plasma exchange, (which puts your blood through a machine and cleans it and then returns it to your body) to give my body some strength. They couldn’t find a visible vein on my arm so they had to do in a vein in my groin; a surgeon used an ultrasound scan to determine where to put it. I hardly felt a thing to be honest because I was just lifeless and so tired.

After 3 days in intensive care, I was moved to an adult ward in a private room. I had 6 plasma exchanges and each of them took 2 hours to do. After each plasma exchange I felt myself getting stronger and I began walking around with the help of my mum. I had visits from my dad every evening and my brother came occasionally! After a week or two I started to eat slowly again with easy food that I could swallow like yogurts and mashed up fruit.

Mr Busby came round and told me what he suspected, "
Myasthenia gravis" a serious muscle weakness condition. I worried how it would affect me and got a bit depressed the more I knew about it.

By this time, it was December and I would have been out buying presents for my friends but I was in hospital. I wondered whether I would be out in time for Christmas Eve as I didn’t want to spend Christmas in hospital, I wanted to spend it at home with my family. Thankfully, I was nearly well enough to go home and agreed to discharge me if I drank a nutritional milkshake and take some steriods everyday. I agreed and they took my feeding tube out from my nose! It was very uncomfortable but I was pleased it was out. I was home on the day before Christmas Eve! Although I was feeling rather isolated despite having all my family and friends at our house and I tried to join in the festive season. I had various check ups over the Christmas holidays. I felt very depressed about the fact I might have
Myasthenia gravis and how I would cope with it. My family and friends reassured me that I would get through it. I was so grateful to hear those words at the time and motivated me to get better!

Myasthenia gravis?

As a result of having ear infections in my right ear that I lost my cochlear implant in, I still underwent operations on my right ear to see what the problem was as Mr Raine was puzzled by it.In November 2003 when I was 18 Mr Raine was undergoing a routine operation for last attempt to find out the problem and he put an ear wick to soak up the ear infection.It was not successful.

The day after the operation, I began to get aches and getting a bit stiff but I put it down to being in a hospital bed! Even a walk around the ward to stretch my legs didn’t help but I wasn't worried about it. I was discharged from hospital and over the next few days, I began to go downhill, it started with my legs being weak and I couldn’t move them very well. I had to swap rooms with my brother because I couldn’t find the strength to get up my bunk bed in my room. I found myself not being able to sleep because I was getting aches all over and the following days each part of me got weaker....I couldn’t eat because I found it hard to swallow food; I couldn’t even lift my head or even stand up without collapsing. My mum slept in the room with me because whenever I needed to go to the toilet, she literally had to lift me up and guide me there. I got very frustrated because I couldn’t do anything myself, I would be in the verge of tears every time I tried to do something as simple as lifting up my head.

A week later, I was still wide awake without a wink of sleep since I left hospital. I spent a Sunday morning watching the rugby world cup on TV with my mum and England had won the world cup. After watching that match, my mum decided enough was enough and called the GP for me the next morning. The GP agreed that I was very poorly and ordered an ambulance to take me back to hospital. I arrived at the hospital with mum a few hours later and they got me settled on a ward and waited for the doctor to come round to give me a check up. I just couldn’t be bothered dealing with anything because I was so weak and wanted to sleep but I couldn’t. My mum had to go home as visiting hours were over and I was distraught at this because I needed a familiar face with me. The first night at the hospital was awful, I was given a sleeping medicine and asked the nurses to make me comfortable because I couldn’t move myself but they walked off and left me to it! I felt so helpless and I just wanted my mum Although one nurse put the buzzer next to me to press if I wanted any help, one of them took it away from me and put it out of reach and I couldn’t even sit up to press it and I cried myself to sleep. My mum came the next morning and made a complaint about it.

The morning doctor came round and checked me out and agreed that I was extremely poorly and sent me to hospital ward after hospital ward but at one point I ended up on an old people’s ward where I stayed for a few days. My mum was disgusted with the hospital because I should have never been on that ward and made yet another complaint. On that particular ward, I was told by a friendly doctor Mr Busby that they needed to get some nutrition into my body and that they needed to put a tube down my nose into my stomach. I was attached to a feed to try and get me feeling a bit better as I hadn't eaten as much as a yougurt in a week and my weight had dropped from 7st to 5st.Mr Busby came back to see me a few days later and I was slightly better and told me I was still very sick and that I would be going to the intensive care unit. Mr Busby thought it may be
Myasthenia Gravis but wasn't sure until further tests.

For now the idea was to get me back on my feet and feeling better!

My 2nd Cochlear Implant

My 2nd operation was shorter than the last. It was November 2000 and I had just finished my mock GCSEs I knew the routine by now when it came to operations, the pre-op appointment and the tests but I never got used to the being put to sleep stage as I’d had so many and I’d hated the cannula needle that they put in your hand to do it. No matter how many operations I had, it got worse. I actually felt fine during this particular operation though because I knew I was going to hear again and I was grateful for the chance.

The hospital was a 3 day stay this time compared to the week I had on the first cochlear implant and I was happy about that because no one likes being in hospital and I was so bored. There wasn’t a lot to do apart from sit around and read a magazine! Mr Raine was also doing this operation on me. I was also pleased that I didn’t have to have my stitches out because he had used dissolvable ones.After a month recovering once again in my silent world, I went back to hospital to get my new external part of the cochlear implant which was a BTE (behind the ear) one. A simple flesh coloured one with a brown coil to match my hair colour. I was quite pleased to have a BTE cochlear implant because with the box one I used to have there was always a risk of catching my wire on something and my BTE one was quite small and petite and out of harms way! I was quite happy with my new cochlear implant and savoured my new sounds! Later on I was given the silver 3G esprit which was even better! I was quite used to the programming now and knew the routine. Being on the adult programme at the cochlear implant centre, I get annual reviews to see how everything is going

Disaster Strikes!

6 years later, in 2000 I suddenly caught an ear infection which might only seem a little thing to anyone but an ear infection can be a disaster for cochlear implantees. It has to be treated as an emergency! I was given ear drops and antibiotics by my cochlear implant doctor Mr Raine but to no avail, the ear infection stayed. Mr Raine decided to put in a few more wicks with cream on in my ear over a few more months. When the infection did'nt go, he decided to do a few exploratory operations but in 2000 when I was 15, Mr Raine asked for my mum's permission during an operation to take the cochlear implant out because it couldn’t be saved because of the ear infection. It was NOT because of the cochlear implant. When I woke up from the operation and my mum told me what had happened, I was very upset because I loved my cochlear implant. I loved listening to my family and friend's voices and I worried that I would never hear them again. I would find out later when i was 19 years old that I had a condtion called Multiple mitochondrial DNA deletions and that my energy genes did'nt work, this was a factor in why I went deaf in the first place and why I had the ear infection so long which led to me losing the cochlear implant.

I was at high school,Year 10 and in the middle of doing my mock GCSE's. I had the fantastic support of the deaf base I had at my high school and they helped me with notes and what the teachers were saying. I had the news that I could get a cochlear implant in my left ear. It would be a thinner magnet in the ear and more modern than the last one. I was sooo pleased about this!! Although, I was back in my silent world for 3 months while I recovered from losing the one on my right ear. I had brilliant help from my family and friends who wrote
notes for me and let me know what was going on. I could also lipread.

The switch on

After 4 weeks I returned to the hospital to collect the cochlear implant and to be programmed. The audiologist handed me a strange looking box device and taught me how to put it on my ear.He then plugged in a wire into the cochlear implant and went back onto the computer and did a series of "beep" tests to determine the pitch and so on.... i remember being very surprised when they switched me on! I wasn't expecting it and i was playing with my toys at the time so the first thing i heard was a clunking sound and then the audiologist said "Laura can you hear me?" and turned my head after I heard it because I didnt know where it was coming from! The audiologist used toys like a macarena shaking to see whether I heard it. I remember hearing my mum talk again for the first time, it wasn't perfect sound but it meant a lot to me and it was very exciting! I wanted to hear more.
After months of several programming sessions, when it was first switched on, it was very echoey and funny sounding! My brain was getting round to hearing sounds again. The hearing started to become clearer as time went on. My listening skills got better as the years went on and I was happy to hear my family and friends voices again and my speech got better too after lots of sessions of speech therapy at the Cochlear implant centre and at my junior school.

My 1st Cochlear Implant

For people who dont know what a cochlear implant is: Here is a link that tells you all about cochlear implants and how they help a deaf person.

I wasn't born deaf, my hearing gradually deteriorated as I grew up and I had various hearing aids from hospital but they never helped. At Easter 1993 I woke up and found myself profoundly deaf when I was 8 yrs old. My parents found out about a device called a Cochlear Implant and tried to find out more about them. My parents also had to try and convince the local council to give us the money for the operation including a front page spread on the local paper. We eventually got the money from the council and the operation could go ahead!

I had lots of tests before the actual operation like blood tests and all the necessary things that needed to be done to see if I was all set to go, it was all very annoying for an 8 year old child but it needed to be done!I was unaware of all the news going on around me because I was in my silent world and wasn’t following anything. My parents kept me up to date by writing notes for me. I was still at junior school while all this was happening and I had a helper at school called Mrs Porter. She wrote notes and helped me with my schoolwork. It was annoying that i had to take time off school to have the operation and stay at home til i was well again. I knew that I would miss my friends from school but I also knew I would soon be back there!

My operation was all set to take place on 24th Feb 1994 at Bradford Royal Infirmary. I was very scared about the operation but my parents knew it was the best thing for me. I remember all the pre-op tests I had before I had the operation and looking around the ward in the hospital I would be staying at. I was a bit naive and didn’t fully know what was going on, my parents wrote me a note "we are going ahead with the operation" I was half excited at the news I might be able to hear again and half worried that I had to have an operation! All my tests were clear so the operation could proceed! I remember getting up very early and our family friends giving me and mum a lift to the hospital. My brother would be staying with them because id be in hospital for a week and my dad was working at the time so he would be there in the evening. After checking in with the nurses on Ward 16, I had my medical history told to them.I spent a few hours waiting, playing in the hospital play area until a nurse came up and gave me a "magic cream" for my hand! I didn’t know it was numbing cream and carried on playing on the sonic hedgehog game. I then went to my room to change into my hospital gown. After an hour of more playing the hospital bed came to take me to surgery. The hospital porter popped me on the bed and off we went and I saw "theatre" above the room we went to which was v scary and my heart started beating v fast. I had my teddy with me for protection and my mum came into theatre with me to hold my hand. One of the doctors took my hand and wiped off the "magic cream" and put a cannula needle in to put me to sleep. Mr Raine was my surgeon. The operation lasted 5 hours and I woke up very groggy with a tight bandage around my head with my right side a bit sore. It was evening and I saw my dad standing in the doorway. When he saw I had woken up, he went to get mum and she told me "go back to sleep you've had a hard day" so I drifted off to sleep again.

Over the next few days I gained strength and had my bandage taken off to reveal the scar of my cochlear implant. I spent 2 more days in hospital to get my stitches taken out it was a bit uncomfortable. Mr Raine gave me one last check up before I went home the next day still in my silent world and for my scar to recover before I received the external part of my cochlear implant.